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Keeping it Together. Axonal Transport to the Synapse and the Effects of Molecular Chaperones in Health and Disease

  • Christopher Sinadinos
  • Amrit Mudher
Chapter

Abstract

Robust intracellular transport along the axon facilitates the delivery and replacement of somally synthesised macromolecules to and from the synapse, and is thus essential for the maintenance of neuronal function. In contact with an intricate cytoskeletal network, multi-subunit motor complexes drive axonal ­transport during journeys through an axonal compartment that is relatively devoid of machineries for de novo protein synthesis and turnover. Very little is known about how these complex transport machineries are assembled and maintained within the axon. In this review, possible roles for molecular chaperone proteins in the ­maintenance of processive, appropriately regulated axonal transport are ­considered. When such transport quality control is compromised, as in the case of ­neurodegenerative proteinopathies, such as Parkinson’s, Alzheimer’s and Huntington’s diseases, pathological axonal transport disruption amidst aberrant protein folding and ­aggregation can result, severing an essential life-line and jeopardising synaptic function. How the stress-induced protein folding and aggregation-blocking functions of molecular chaperones are overcome in a range of pathological circumstances is also considered.

Keywords

Molecular Chaperone Axonal Transport Motor Complex Axonal Swelling Torsional Dystonia 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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© Springer Science+Business Media, LLC 2011

Authors and Affiliations

  1. 1.Southampton Neuroscience Group, School of Biological SciencesUniversity of SouthamptonSouthamptonUK

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